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今天给大家带来的内容是
【肺朗格汉斯细胞组织细胞增多症】
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图1 胸片正位
图2 胸部CT肺窗
图3 胸部CT肺窗
图4 胸部CT肺窗
图5 胸部CT肺窗
Posteroanterior chest radiographs reveal diffuse reticular opacities and small nodules throughout the lungs.
胸片显示全肺弥漫性网格影及小结节影。
Axial CT scans in lung window setting demonstrate widespread small, bizarre shaped lung cysts with resultant parenchymal destruction.
胸部CT横断面肺窗显示广泛分布的、小而不规则肺囊肿，致肺实质破坏。
All lobes were involved with relative sparing of the costophrenic angles. 所有肺叶均受累，而肋膈角则相对未受累。
Pulmonary Langerhans cell histiocytosis
肺朗格汉斯细胞组织细胞增多症
Video-assisted thoracoscopic surgery was performed to obtain diagnostic wedge biopsy specimens of the right upper and right lower lobes.
行电视辅助胸腔镜手术获取右上肺及下肺楔形活检样本。
Pathologic findings were consistent with pulmonary Langerhans cell histiocytosis (LCH). 病理结果与LCH一致。
Pulmonary LCH refers to LCH isolated to the respiratory system, notably the lungs.
肺LCH是指LCH单独累及呼吸系统， 特别是肺。
This rare disease is found almost exclusively in cigarette smokers, which supports the theory of antigen exposure, since cigarette smoke contains thousands of known antigens.
该少见疾病几乎仅见于吸烟者，这支持了抗原暴露理论，因为烟雾含有数千种已知抗原。
The radiologic findings of pulmonary LCH vary depending on the stage of the disease at diagnosis.
肺LCH的放射学表现取决于疾病的进展阶段。
Diffuse bilateral ill-defined nodules will be seen in early stages. Because the disease has an inhalational component, the middle and upper lung zones are involved to a greater extent than the lung bases.
早期可见弥漫性双侧边界不清结节。由于该疾病含有吸入性成分，中上肺区比肺底部受累范围更大。
It is thought that these nodules undergo cystic degeneration as the disease progresses, and so a reticular pattern begins to predominate on chest radiographs as the numerous cystic walls are superimposed on one another.
随着病情进展，这些结节发生囊变。由于许多囊壁相互重叠，因此在胸片上开始以网格影为主。
The cysts and residual parenchyma can undergo fibrosis over time and eventually lead to changes of honeycombing.
随时间推移，囊肿和残余肺实质发生纤维化，最终导致蜂窝肺改变。
High-resolution CT plays an important role in the evaluation of pulmonary LCH. The diagnosis can be easily made with high-resolution CT by showing both ill-defined nodules and cysts in a heavy smoker.
HRCT在肺LCH的评估中起着重要作用。HRCT可对重度吸烟者的边界不清结节和囊肿清晰显示并作出诊断。
However, the diagnostic accuracy of high-resolution CT falls short when only nodules or cysts alone are present. Most of these cases are confirmed with lung biopsy.
然而，当结节或囊肿单独出现时，HRCT的诊断准确率下降。多数病例经肺活检确诊。
Patients with pulmonary LCH have variable and uncertain clinical courses. Up to one-half will show clinical and radiographic stability, while up to 25% will demonstrate spontaneous regression. The remaining 25% can have continued cystic replacement of parenchyma that may progress to end-stage lung disease.
肺LCH患者的临床病程多变且不确定。高达一半的患者临床和影像学稳定，而高达25%的患者可自发转归。剩下的25%肺实质囊性变，可能进展为终末期肺病。 reticular opacities：网格影 parenchymal：实质 costophrenic angles：肋膈角 exclusively：仅 antigen：抗原 ill-defined：边界不清的
鸣谢：Geewon Lee, Ji-Won Lee / Pusan National University Hospital
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